Idiopathic Non-cirrhotic Portal Hypertension and Close Entities: a Need for Clarifying Terminology

Idiopathic non-cirrhotic portal hypertension is described with a very heterogeneous terminology depending on the geographic origin of the patients and on the possible underlying causes of the disease. We aim here to review the current nomenclature and to the pathophysiological origins of this entity. In the past, a relevant number of definitions of this condition have been used in the literature, suggesting the presence of several distinct diseases. However, recent findings strongly suggest that there are some common denominators for this disease. Therefore, efforts should be made to develop a unified terminology encompassing the very essential features from clinical, histologic, imaging, laboratory and elastographic data. These findings suggest that the common localization of the disease is the portal and the sinusoidal compartment of the liver; therefore, a novel definition should be elaborated.